Malabsorption Syndromes

Malabsorption Syndromes
Nursing management of pediatric patients with Genetic disorders
 
|
 
The Child with
Gastrointestinal Dysfunction
Dr. Moustafa Ali
 
After mastering the contents of this lecture, you should be able to:
1.
Identify the common problems affecting the gastrointestinal system
during childhood
2.
Provide nursing care to a child with malabsorption syndrome by
implementing the nursing process
3.
Identify nutritional therapies for the child with a malabsorption
syndrome
O B J E C T I V E S
Malabsorption Syndromes
These include disorders with impaired digestion and or
absorption.
Classification is done according to anatomic and or
biochemical defect it has:
(continued)
Four characteristics
Seatoria (Fatty, Foul, Frothy bulky stools)
General malnutrition
Abdominal distention
Secondary Vitamin deficiencies
 
Malabsorption Syndromes
Include diminished or absent enzymes such as:
1.
Cystic fibrosis: 
Pancreatic enzyme is absent
2.
Biliary liver disease: 
bile flow is affected (Artesia of the bile duct)
3.
Lactase deficiency: 
Congenital or secondary Lactase intolerance
Digestive defects
Conditions in which the intestinal mucosal transport system is impaired.
Primary defect (
Celiac disease
) or
Secondary to inflammatory disease in the bowel leading to impaired
absorption, because bowel mobility is accelerated such as Ulcerative
Colitis
Obstructive Disorders such as 
Hirschsprung's Disease 
can cause
secondary malabsorption from 
Enterocolitis
.
Anatomic defects
: extensive resection of the bowel or short bowel
syndrome.
 
Absorptive defects
Celiac disease (CD)
Gluten
 – induced enteropathy, gluten sensitive
enteropathy (
GSE
) Celiac sprue. It is secondary to cystic
fibrosis to cause malabsorption in children
(continued)
Incidence
 1:4000 but it appears to be declining may be due
to environmental factors. ++ in 
Europe
 and rare in 
America
and is rarely reported in 
Asians
 or 
blacks
Cause
 is unknown
There is 
no inherited 
predisposition with an influence by
environmental factors
 
Incidence
Intolerance of 
gluten
 a protein found in Wheat, Barley, Rye and
Oats
Gluten consists of:
Glutenin
Gliadin
: Susceptible individuals are 
unable to digest  
gliadin
fraction resulting in 
accumulation
 of a toxic substance that
damage the 
mucosal cells 
leading to 
villi atrophy
, and
reducing the 
surface for absorption 
in the small intestine
Pathophysiology
C/M noticed 
at 
3 – 6 months 
following introduction
of gluten –  containing grains into diet Age usually 
9 -
12 months
Diagnosis
villi of the intestine
C/M insidious and chronic they include:
Impaired fat absorption:
 Steatorrhea and exceedingly foul smelling stools
 
Diagnosis
Impaired absorption of nutrients leading to:
Malnutrition
Muscle wasting (legs and Buttocks)
Anemia
Anorexia
Abdominal distention
Diagnosis
Impaired absorption of nutrients leading to:
Behavioral changes:
Irritability
Fretfulness
Uncooperativeness
Apathy
Diagnosis
Celiac crisis (in very young infants):
Present as acute, severe episodes of profuse watery diarrhea
and vomiting
Diagnosis
Celiac crisis (in very young infants):
 May be precipitated by:
Infections (especially in the GI)
Prolonged fluid and electrolyte depletion
Emotional disturbances
Diagnosis
Celiac crisis (in very young infants):
First incidence may be failure to thrive and diarrhea
 Definitive diagnosis is by Jejunal biopsy that shows atrophy
and changes in the small intestine
 The improvement after gluten free diet is also diagnostic
which occur within a day or two
Diagnosis
Celiac crisis (in very young infants):
Change in personality, weight gain, increase in the appetite,
and decrease in diarrhea and steatorrhea occur after several
days or weeks
 Histological and or clinical relapse follow  gluten
reintroduction
Diagnosis
Dietary management:
Restricted Gluten diet
Corn and rice are given instead
 
 
Supplemental vitamins, Iron and Calories are given for severe
malnutrition
Treatment
NO
It is a chronic disease
Most severe symptoms occur in early childhood and
again in adult life
Strict dietary avoidance can prevent symptoms and
may minimize the risk of developing 
lymphoma
 one
of the most serious complications
Prognosis
To 
help
 the parents and the child to adhere to the prescribed
diet
To 
explain
 the disease process to them
 
Advice
 parents to read all ingredients on labels to 
avoid
hidden sources of 
Gluten
 as in “Hydrolyzed vegetable
protein” e.g. 
Hotdogs
, 
Pizza
 and 
Spaghetti
, Instant soup
these are not allowed.
Nursing considerations
In 
severe
 cases Disaccharides digestion is impaired especially in
relation to 
Lactose
, so temporary Lactose – free diet given, and (milk
products) are 
restricted
 from the diet
Diet should be high in 
calories
 and 
proteins
 with simple
carbohydrate
, 
fruits
 and 
vegetables
 and low fat.
The 
rough vegetables 
and fruits are 
avoided
 until acute
inflammation heals
Any faulty in the diet will establish the  previous signs and symptoms.
Nursing considerations
1.
Identify the common problems affecting the gastrointestinal system
during childhood?
2.
Provide nursing care to a child with malabsorption syndrome by
implementing the nursing process?
3.
Identify nutritional therapies for the child with a malabsorption
syndrome?
Evaluation
Hockenberry, M. J., Wilson, D., (2015) Wong’s Nursing Care of Infants and Children 10th
Edition.  Mosby, St Louis MO.
Pillitteri, A.: Maternal And Child Health Nursing: care of the childbearing and childrearing
family, 2010, 6th edition
References
Thank
 
you
You can find me at:
mustafa11ali@yahoo.com
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The nursing management of pediatric patients with malabsorption syndromes, including identification of common gastrointestinal problems, implementation of the nursing process, and nutritional therapies.

  • malabsorption syndromes
  • pediatric nursing
  • genetic disorders
  • gastrointestinal dysfunction
  • nursing process
  • nutritional therapies

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  1. Malabsorption Syndromes Nursing management of pediatric patients with Genetic disorders | The Child with Gastrointestinal Dysfunction Dr. Moustafa Ali

  2. O B J E C T I V E S After mastering the contents of this lecture, you should be able to: 1. Identify the common problems affecting the gastrointestinal system during childhood 2. Provide nursing care to a child with malabsorption syndrome by implementing the nursing process 3. Identify nutritional therapies for the child with a malabsorption syndrome

  3. Malabsorption Syndromes These include disorders with impaired digestion and or absorption. Classification is done according to anatomic and or biochemical defect it has: (continued)

  4. Malabsorption Syndromes Four characteristics Seatoria (Fatty, Foul, Frothy bulky stools) General malnutrition Abdominal distention Secondary Vitamin deficiencies

  5. Digestive defects Include diminished or absent enzymes such as: 1. Cystic fibrosis: Pancreatic enzyme is absent 2. Biliary liver disease: bile flow is affected (Artesia of the bile duct) 3. Lactase deficiency: Congenital or secondary Lactase intolerance

  6. Absorptive defects Conditions in which the intestinal mucosal transport system is impaired. Primary defect (Celiac disease) or Secondary to inflammatory disease in the bowel leading to impaired absorption, because bowel mobility is accelerated such as Ulcerative Colitis Obstructive Disorders such as Hirschsprung's Disease can cause secondary malabsorption from Enterocolitis. Anatomic defects: extensive resection of the bowel or short bowel syndrome.

  7. Celiac disease (CD) Gluten induced enteropathy, gluten sensitive enteropathy (GSE) Celiac sprue. It is secondary to cystic fibrosis to cause malabsorption in children (continued)

  8. Incidence Incidence 1:4000 but it appears to be declining may be due to environmental factors. ++ in Europe and rare in America and is rarely reported in Asians or blacks Cause is unknown There is no inherited predisposition with an influence by environmental factors

  9. Pathophysiology Intolerance of gluten a protein found in Wheat, Barley, Rye and Oats Gluten consists of: Glutenin Gliadin: Susceptible individuals are unable to digest gliadin fraction resulting in accumulation of a toxic substance that damage the mucosal cells leading to villi atrophy, and reducing the surface for absorption in the small intestine

  10. Diagnosis C/M noticed at 3 6 months following introduction of gluten containing grains into diet Age usually 9 - 12 months villi of the intestine

  11. Diagnosis C/M insidious and chronic they include: Impaired fat absorption: Steatorrhea and exceedingly foul smelling stools

  12. Diagnosis Impaired absorption of nutrients leading to: Malnutrition Muscle wasting (legs and Buttocks) Anemia Anorexia Abdominal distention

  13. Diagnosis Impaired absorption of nutrients leading to: Behavioral changes: Irritability Fretfulness Uncooperativeness Apathy

  14. Diagnosis Celiac crisis (in very young infants): Present as acute, severe episodes of profuse watery diarrhea and vomiting

  15. Diagnosis Celiac crisis (in very young infants): May be precipitated by: Infections (especially in the GI) Prolonged fluid and electrolyte depletion Emotional disturbances

  16. Diagnosis Celiac crisis (in very young infants): First incidence may be failure to thrive and diarrhea Definitive diagnosis is by Jejunal biopsy that shows atrophy and changes in the small intestine The improvement after gluten free diet is also diagnostic which occur within a day or two

  17. Diagnosis Celiac crisis (in very young infants): Change in personality, weight gain, increase in the appetite, and decrease in diarrhea and steatorrhea occur after several days or weeks Histological and or clinical relapse follow gluten reintroduction

  18. Treatment NO Dietary management: Restricted Gluten diet Corn and rice are given instead Supplemental vitamins, Iron and Calories are given for severe malnutrition

  19. Prognosis It is a chronic disease Most severe symptoms occur in early childhood and again in adult life Strict dietary avoidance can prevent symptoms and may minimize the risk of developing lymphoma one of the most serious complications

  20. Nursing considerations To help the parents and the child to adhere to the prescribed diet To explain the disease process to them Advice parents to read all ingredients on labels to avoid hidden sources of Gluten as in Hydrolyzed vegetable protein e.g. Hotdogs, Pizza and Spaghetti, Instant soup these are not allowed.

  21. Nursing considerations In severe cases Disaccharides digestion is impaired especially in relation to Lactose, so temporary Lactose free diet given, and (milk products) are restricted from the diet Diet should be high in calories and proteins with simple carbohydrate, fruits and vegetables and low fat. The rough vegetables and fruits are avoided until acute inflammation heals Any faulty in the diet will establish the previous signs and symptoms.

  22. Evaluation 1. Identify the common problems affecting the gastrointestinal system during childhood? 2. Provide nursing care to a child with malabsorption syndrome by implementing the nursing process? 3. Identify nutritional therapies for the child with a malabsorption syndrome?

  23. References Hockenberry, M. J., Wilson, D., (2015) Wong s Nursing Care of Infants and Children 10th Edition. Mosby, St Louis MO. Pillitteri, A.: Maternal And Child Health Nursing: care of the childbearing and childrearing family, 2010, 6th edition

  24. Thankyou You can find me at: mustafa11ali@yahoo.com

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